Left Ventricular Noncompaction—A Systematic Review of Risk Factors in the Pediatric Population

Abstract

Left ventricular noncompaction (LVNC) is a heterogeneous, often hereditary group of diseases, which may have diverse clinical manifestations. This article reviews the risk factors for unfavorable outcomes of LVNC in children, as well as discuss the diagnostic methods and the differences between pediatric and adult LVNC. Through a systematic review of the literature, a total of 1983 articles were outlined; 23 of them met the inclusion criteria. In echocardiography the following have been associated with adverse outcomes in children: Left ventricular ejection fraction, end-diastolic dimension, left ventricular posterior wall compaction, and decreased strains. T-wave abnormalities and increased spatial peak QRS-T angle in ECG, as well as arrhythmia, were observed in children at greater risk. Cardiac magnetic resonance is a valuable tool to identify those with systolic dysfunction and late gadolinium enhancement. Genetic testing appears to help identify children at risk, because mutations in particular genes have been associated with worse outcomes. ECG and imaging tests, such as echocardiography and magnetic resonance, help outline risk factors for unfavorable outcomes of LVNC in children and in identifying outpatients who require more attention. Refining the current diagnostic criteria is crucial to avoid inadequate restrain from physical activity.

Reduced Systolic Function and Not Genetic Variants Determine Outcome in Pediatric and Adult Left Ventricular Noncompaction Cardiomyopathy

“…Left ventricular dilation and systolic dysfunction were less strong predictors for survival than New York Heart Association class III/IV and cardiovascular complications at presentation (27). According to our results, reduced LV systolic function is the most important prognostic factor for clinical outcome (28,29). Asymptomatic patients with normal echocardiography mostly remain with normal cardiac function during long-term follow-up.…”

Left ventricular noncompaction: a disorder with genotypic and phenotypic heterogeneity—a narrative review

“…A recent meta-analysis estimates the prevalence, clinical presentation, natural history, and prognosis of LVNC. The cardiovascular mortality rate for children is 4.3 per 100 live births per year (151). The cardiovascular mortality rate for adults is 1.9 per 100 live births per year (152).…”

Primary cardiomyopathies in childhood: clinical and diagnostic features (literature review)

“…Например, ГКМП, в основе которой лежат саркомерные нарушения, может на определенном этапе заболевания переходить в ДКМП. Некомпактный миокард можно увидеть изолированно или в комбинации с другими фенотипами, а именно с фенотипом ГКМП или ДКМП [11].…”

“…По мнению ряда авторов, истинная заболеваемость НКМП может быть выше, чем предполагалось ранее, т.к. стандартизированный подход к постановке этого диагноза появился относительно недавно [9,11]. J.L.…”